Extra-adrenal pheochromocytoma with initial symptom of haemoptysis: a case report and review of literature

BMC Surg. 2021 Jan 6;21(1):13. doi: 10.1186/s12893-020-01038-6.

Abstract

Background: Pheochromocytoma is a catecholamine-secreting tumour that leads to various symptoms. Haemoptysis is rarely caused by a pheochromocytoma occurring outside the bronchus or thoracic cavity. Here, we report the case of an extra-adrenal abdominal pheochromocytoma initially manifesting as haemoptysis/dyspnoea during exercise without classic symptoms.

Case presentation: A 22-year-old man with a history of severe dyspnoea experienced difficulties in breathing following a marathon owing to haemoptysis that required ventilator management 1 year before presentation. His father had undergone surgery for ectopic pheochromocytoma. Computed tomography (CT) revealed a 30-mm tumour between the inferior vena cava and pancreatic head while urinalysis revealed abnormally high noradrenaline levels. He was clinically diagnosed with an extra-adrenal abdominal ectopic pheochromocytoma. After controlling blood pressure, surgery was performed, and the tumour was successfully removed. Histopathology revealed chromogranin A (+), synaptophysin (+), S100 protein (+), and MIB-1 index of 1%. Therefore, the patient was finally diagnosed with extra-adrenal abdominal ectopic pheochromocytoma.

Conclusions: Haemoptysis is a rare manifestation of abdominal ectopic paraganglioma. Prompt consideration of pheochromocytoma/paraganglioma when patients experience haemoptysis without any other possible aetiology may prevent inappropriate diagnosis and treatment and ultimately fatalities.

Keywords: Extra-adrenal abdominal pheochromocytoma; Haemoptysis; Paraganglioma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenal Gland Neoplasms* / diagnosis
  • Adrenal Gland Neoplasms* / surgery
  • Adult
  • Hemoptysis* / etiology
  • Humans
  • Male
  • Neoplasm Recurrence, Local
  • Pheochromocytoma* / diagnosis
  • Pheochromocytoma* / surgery
  • Tomography, X-Ray Computed
  • Young Adult